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Heart Transplants in Children

Orthotopic cardiac transplantation is the process of removing a person's failing heart and replacing it with a suitable donor heart from a person who has been declared clinically brain dead.

Orthotopic refers to placing the new heart in the chest and contrasts the procedure from "heterotopic" transplantation (placing the transplanted heart elsewhere in the body but keeping the patient’s original heart in place).

Once a patient has been accepted for transplantation, he or she is placed on a nationwide list. Based on the urgency, blood type, size, and time on the list, donor hearts are offered to a transplant center for a particular patient.

Once a donor organ is accepted, a team travels to the donor hospital to retrieve the heart. The heart is cooled with a special solution that stops the heart and preserves it. The heart is placed in cold saline and transported in an ice cooler back to the hospital where the patient getting the heart is waiting.

The patient's chest is opened and he or she is placed on a heart-lung machine (cardiopulmonary bypass). The failing heart is removed and the new donor heart is sutured into place.

In most cases, a bigger heart from a donor can be used since the original failing heart may be enlarged as a consequence of heart failure.

For patients who have not had prior surgery, the transplant operation typically takes about five hours. Post-operative recovery typically involves a 10- to 14-day hospital stay.

The immediate risks of transplantation include the usual risks of open-heart surgery. The risks may be increased based on the patient's pre-operative condition.

Acute graft failure either from pre-formed antibodies (rejection) or from primary graft dysfunction is quite rare in the current era.

The early mortality is less than 5 percent for primary transplants in patients in good pre-operative condition.

The most frequent early complications include renal (kidney) dysfunction, infection and bleeding.

Temporary right ventricular dysfunction may occur due to high pressure in the lungs that many recipients may have as a result of longstanding heart failure or, more typically, due to ischemic effects of organ procurement.

Patients awaiting transplantation may be on anticoagulants to prevent clots from forming in the heart. They may also be on medications to prevent dangerous rhythms from occurring, which are more frequent in failing hearts.

If the pulmonary resistance is significantly elevated or the heart function too poor on its own, patients may remain on intravenous (IV) medications in the hospital while awaiting transplantation.

Mechanical circulatory support may also be employed to support a patient awaiting transplantation.

Literature on transplantation is provided to all patients and their families during the evaluation process.

Potential heart transplant recipients are usually identified by their surgeon or cardiologist. The patient and family are presented the option of transplantation and educated about the process and care involved.

The evaluation process involves blood work, further studies of heart pressures and function, if needed, a general medical evaluation, and evaluation of the patient and the family's social situation and support system.

If all are in agreement, the patient is presented at a multidisciplinary transplant meeting and a group decision is made. Not every patient who may need a transplant is acceptable for transplantation. Reasons that some patients are not listed for transplantation include:

Patient / family desire

Medical contraindications

Other contraindications

Heart transplantation is considered as a last resort for patients with end-stage heart disease who may have no other surgical or medical therapies available.

Generally, people listed for heart transplantation have a life expectancy of less than one year. Additionally, these patients often have significant limitations of their activity and lifestyle prior to transplantation.

The goal of heart transplantation is to return the patient to a state of functionality with the least amount of limitations and to optimize quality of life.

The more common causes of end-stage heart disease requiring a transplant in children include:

Cardiomyopathy -- a progressive deterioration of the function of the heart muscle

Congenital heart defects that are not amenable to further correction

Palliation

In adults, ischemic heart disease (coronary artery disease) is the most common reason for heart transplantation. Transplant coronary artery disease may be a reason for considering re-transplantation in children.

More than 400 heart transplants are performed in pediatric patients each year, according to the International Society for Heart and Lung Transplantation.

Wait-list time for transplantation varies depending on a patient's size, blood type and waiting status. Typically a patient would wait weeks to months before an appropriate donor offer is available. It is not uncommon to wait for more than a year.

Once a suitable donor heart is available, a patient generally has two to four hours to get to the hospital and be prepared for surgery.

Heart transplantation should be considered a palliative and not a curative operation. While most patients return to good functional status, transplanted hearts do not last as long as a normal native heart in many patients.

Survival rates have continued to improve with experience, better techniques and medications, and improved rejection surveillance and immunosuppression.

Patients must remain on multiple medications for the rest of their lives. One type of medication, immunosuppressants, must be taken forever to help prevent against rejection. Some of these medications can have significant side effects, requiring other medicines for treatment. It is not unusual for a heart transplant recipient to be on 10 to 12 medicines at one time. It is critical that the patient adheres strictly to these regimens to avoid the many potential complications of transplantation.

Transplant patients are at risk for infection, and the development of lymphoproliferative disorders (a form of cancer) because of these medications. Rejection may also occur. The dosage of medicines has to be changed at times based on blood tests, evidence of infection or cancer, or evidence of rejection. Close follow-up with the transplant team and doctors is needed and it is not unusual for patients to come back to the hospital often for these assessments.

An aggressive form of coronary artery disease has also been described in transplanted hearts that may represent a form of chronic rejection.

Patients may undergo retransplantation if the transplanted heart starts to fail. Repeat transplants, however, do not always do as well, and some patients may not qualify for a retransplant due to medical or social reasons.

The overall survival for orthotopic transplantation is currently around 90 percent at one year and 75 percent at five years. About 50 percent of transplant recipients make it out more than 14 years.